1. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry
- Author
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Bent Bruun Kristensen, Irene Lang, Marion Delcroix, Miguel Ángel Gómez-Sánchez, Repke J. Snijder, Paul Bresser, Marius M. Hoeper, Adam Torbicki, Xavier Jaïs, Eckhard Mayer, A. Hamid, Gérald Simonneau, Marco Morsolini, Jerzy Lewczuk, Joanna Pepke-Zaba, Andrea Maria D'Armini, Pavel Jansa, Gabor Kovacs, Rudolf Speich, David Ambroz, Joan Albert Barberà, Marc de Perrot, Carmen M. Treacy, Iveta Simkova, Sean Gaine, Other departments, University of Zurich, and Pepke-Zaba, J
- Subjects
Endothelin Receptor Antagonists ,Male ,medicine.medical_specialty ,New York Heart Association Class ,Internationality ,Vena Cava Filters ,medicine.medical_treatment ,Hypertension, Pulmonary ,610 Medicine & health ,Endarterectomy ,Scintigraphy ,Riociguat ,2705 Cardiology and Cardiovascular Medicine ,2737 Physiology (medical) ,Recurrence ,Risk Factors ,Physiology (medical) ,Internal medicine ,medicine ,Pulmonary angiography ,Humans ,Prostaglandins I ,Prospective Studies ,Registries ,Aged ,Lung ,medicine.diagnostic_test ,business.industry ,Incidence ,Venous Thromboembolism ,Middle Aged ,Phosphodiesterase 5 Inhibitors ,medicine.disease ,Pulmonary hypertension ,Surgery ,Natural history ,medicine.anatomical_structure ,Chronic Disease ,Cardiology ,Female ,10029 Clinic and Policlinic for Internal Medicine ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - Abstract
Background— Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. Methods and Results— The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%– 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension–targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. Conclusions— Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension–targeted treatments.
- Published
- 2011